cardiovascularКардиоваскулярная терапия и профилактикаCardiovascular Therapy and Prevention1728-88002619-0125«SILICEA-POLIGRAF» LLC10.15829/1728-8800-2012-4-79-84cardiovascular-1878Research ArticleКЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯ И РЕГИСТРЫCLINICAL TRIALS AND REGISTRIESСовременная терапия легочной артериальной гипертензии: анализ данных Северо-Западного регистраContemporary treatment of pulmonary arterial hypertension: the North-West Registry data analysisГончароваН. С.GoncharovaN. S.ns.goncharova@gmail.comКазымлыА. В.KazymlyA. V.ns.goncharova@gmail.comНаймушинА. В.NaimushinA. V.ns.goncharova@gmail.comМоисееваО. М.MoiseevaO. M.omoiseeva@front.ruФГБУ «Федеральный центр сердца, крови и эндокринологии имени В. А. Алмазова», Санкт-ПетербургV. A. Almazov Federal Centre of Heart, Blood, and Endocrinology, St. Petersburg2012200820121147984Copyright © Гончарова Н.С., Казымлы А.В., Наймушин А.В., Моисеева О.М., 20122012Гончарова Н.С., Казымлы А.В., Наймушин А.В., Моисеева О.М.Goncharova N.S., Kazymly A.V., Naimushin A.V., Moiseeva O.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://cardiovascular.elpub.ru/jour/article/view/1878

Цель. В проспективном исследовании показать влияние конвенциональной и специфической терапии на течение заболевания и выживаемость больных легочной артериальной гипертензией (ЛАГ). Материал и методы. В наблюдение включены 124 пациента (средний возраст – 38,2±13,7 лет; М/Ж – 34/78): 31 пациент с идиопатической ЛАГ (ИЛАГ), 52 – с синдромом Эйзенменгера, 17 пациентов с неоперабельной хронической тромбоэмболической ЛАГ, 9 — с ЛАГ на фоне коррегированых врожденных пороков сердца, 6 — с ЛАГ, ассоциированной с системной склеродермией и 6 пациентов с ЛАГ, ассоциированной с ВИЧ-инфекцией. Результаты. Совокупная выживаемость за год всех пациентов регистра составила 94%, трехлетняя – 75%. 42,7% больных получали терапию антагонистами кальция при отсутствии данных о катетеризации правых камер сердца и без проведения вазореактивного тестирования. 40,3% пациентов получали ЛАГ-специфическую терапию, из них 64,5% – пациенты с ИЛАГ и 21% – с синдромом Эйзенменгера. Назначение ЛАГ-специфической терапии увеличивало время до наступления летального исхода. Заключение. Прогноз больных ЛАГ зависит от своевременности назначения специфической монотерапии и возможности применения комбинированной терапии. Создание единого национального регистра позволит оценить реальную потребность в специфической терапии и планировать расходы на приобретение лекарств.

Aim. Using the prospective Registry data, to assess the effects of conventional and specific therapy on the clinical course and survival of the patients with pulmonary arterial hypertension (PAH). Material and methods. The study included 124 patients (mean age 38,2±13,7 years; 34 men and 78 women): 31 with idiopathic PAH (IPAH), 52 with Eisenmenger syndrome, 17 with inoperable chronic thromboembolic pulmonary hypertension, 9 with PAH and corrected congenital heart disease, 6 with PAH and systemic scleroderma, and 6 with PAH and HIV infection. Results. The cumulative one-year and three-year survival rates were 94% and 75%, respectively. Irrespective of the absence of right heart catheterisation and vasoreactive testing, 42,7% of the patients were treated with calcium antagonists. PAH-specific therapy was administered to 40,3% of the participants (64,5% and 21% of those with IPAH and Eisenmenger syndrome, respectively). PAH-specific therapy was associated with an increase in survival time. Conclusion. In PAH patients, the prognosis is linked to early administration of specific monotherapy and possible combination therapy. Developing a national registry of pulmonary hypertension will facilitate the assessment of the real-world demand for specific therapy and the related costs.

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The authors declare that there are no conflicts of interest present.